This protocol addresses primary central nervous system lymphoma presenting as primary vitreoretinal lymphoma (PVRL) — where intraocular vitreoretinal infiltration is the exclusive site of disease at presentation.
PVRL may represent isolated intraocular involvement or occur alongside brain or meningeal disease. When vitreoretinal infiltration is the sole detected site, specific management considerations apply given the risk of CNS dissemination.
Initial treatment of PVRL is generally approached in the same way as standard PCNSL, with systemic polychemotherapy forming the backbone for patients who are fit to receive it. For patients in whom systemic treatment is not appropriate, intraocular local treatment represents a valid alternative approach.
DOI: 10.1093/neuonc/noac196
Intraocular infiltration can be the exclusive site of disease at presentation, referred to as primary vitreoretinal lymphoma (PVRL) or as a part of PCNSL with concomitant brain or meningeal disease.
HD-MTX-based chemotherapy seems to improve OS in PVRL (level C), though local relapses occur frequently.
Nevertheless, given the high risk of relapse in the CNS, and the improved OS after systemic treatment in the most recent series, most experts consider that initial treatment of PVRL should not differ from that of PCNSL ie, high-dose MTX-based polychemotherapy followed, or not, by consolidation treatment to eradicate the possible concomitant microscopic disease in the brain and in the CSF in patients fit to receive such treatment.
Local treatment (intravitreal immuno/chemotherapy or ocular RT) is a valid approach for patients with systemic chemotherapy contraindications or for elderly patients with relapsing intraocular disease (Good Practice Point).
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